Pulmonary alveolar proteinosis (PAP) is a rare lung condition with a primary or acquired form of macrophage dysfunction that results in accumulation of amorphous material in the alveoli. This results in impaired oxygen transfer across the alveolar membrane causing dyspnea. Treatment involves physical removal of the amorphous material by whole-lung lavage (WLL). Despite the increasing experience with the use of recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF) in autoimmune PAP, WLL remains the mainstay of therapy to alleviate symptoms.

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Management of Pulmonary Alveolar Proteinosis

  • Chakravarthy B. Reddy

摘要

Pulmonary alveolar proteinosis (PAP) is a rare lung condition with a primary or acquired form of macrophage dysfunction that results in accumulation of amorphous material in the alveoli. This results in impaired oxygen transfer across the alveolar membrane causing dyspnea. Treatment involves physical removal of the amorphous material by whole-lung lavage (WLL). Despite the increasing experience with the use of recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF) in autoimmune PAP, WLL remains the mainstay of therapy to alleviate symptoms.